TESI DOCTORAL Anna Sabaté Rotés Directors de Tesi

109 Pages·2014·1.26 MB·English

TESI DOCTORAL

RESTORATION OF PULMONARY VALVE COMPETENCE IN
PATIENTS WITH REPAIRED TETRALOGY OF FALLOT, SHORT
AND LONG-TERM EXPERIENCE

Anna Sabaté Rotés

Directors de Tesi:
Dr. Jaume Casaldàliga Ferrer
Prof. Antonio Carrascosa Lezcano

Departament de Pediatria, Facultat de Medicina
Universitat Autònoma de Barcelona
2014

TESI DOCTORAL

RESTORATION OF PULMONARY VALVE COMPETENCE IN
PATIENTS WITH REPAIRED TETRALOGY OF FALLOT, SHORT
AND LONG-TERM EXPERIENCE

Departament de Pediatria, Facultat de Medicina
Universitat Autònoma de Barcelona
2014

Directors de Tesi:
Dr. Jaume Casaldàliga Ferrer

Prof. Antonio Carrascosa Lezcano

Doctoranda:
Anna Sabaté Rotés

ACKNOWLEDGEMENTS

To Dr. Jaume Casaldàliga Ferrer and Prof. Antonio Carrascosa Lezcano who gave me
all their support throughout this research project. And to Harold M. Burkhart MD and
Benjamin W. Eidem MD who mentored me during my advanced training at Mayo
Clinic.

To Christopher Mc Leod MD PhD who guided me through the arrhythmology in
congenital heart diseases.

To David J. Driscoll MD, Jonathan Johnson MD and Thomas G. Allison PhD for
enhancing my training in exercise testing in congenital heart diseases.

To Hector Villarraga MD for teaching me the cutting-edge novel echocardiographic
technology and techniques; and to Chelsea Reece RDCS for tracing all strain curves
anew for the interobserver analysis.

To Carole A. Warnes MD, Joseph A. Dearani MD, Heidi M. Connolly MD, Hartzell V.
Schaff MD and Crystal R. Bonnichsen MD for critically reviewing and improving the
manuscripts and setting an example on how multidisciplinary research collaboration
should be.

To Judy Lenoch and Laurie M. Barr who assisted with the data management.

To Zhuo Li MS, David Hodge MS and Jordan Rosedahl BA who collaborated in the
statistical analysis.

To Carl Clingman for creating the surgical illustrations included in this Thesis.

To all teachers and statisticians at the Center for Clinical and Translational Science
(CCaTS), who taught towards my Certificate in Research at Mayo Clinic and assisted in
several aspects of these projects.
To Fundación Alfonso Martin Escudero who supported me during the first half of my
stay at Mayo Clinic.

To Fundació La Caixa who supported me during the second half of my stay at Mayo
Clinic.

To my colleagues at Hospital Vall d’Hebron who provided me with a solid knowledge
in preparation for my career.

To my colleagues from other institutions who broadened my horizons.

To my friends, for being there, so close even in the distance.

To my parents, Isabel and Xavier, and my brother Xavi, for their unconditional support
and trust.

To my family, Eduard who is the best partner for any endeavor and without whom I
would have never gone that far, and the little one kicking in my tummy who has
accompanied me during the last 9 months and hopefully for the rest of my life.
CONTENTS

C
ONTENTS

Tesi Doctoral – Anna Sabaté Rotés | i
CONTENTS

Tesi Doctoral – Anna Sabaté Rotés | ii
CONTENTS
ABBREVIATIONS ................................................................................................................ 1

INTRODUCTION .................................................................................................................. 3

I.
  Description of the disease ...................................................................................... 5

II.
  Surgical repair ....................................................................................................... 6

RATIONALE FOR THIS RESEARCH, HYPOTHESIS AND AIMS ................................................ 9

I.
  Rationale for this Research .................................................................................. 11

1.-
  Indications for Pulmonary Valve Replacement .............................................. 11

2.-
  Surgical approach ........................................................................................... 14

3.-
  Arrhythmia management ................................................................................ 14

4.-
  Clinical implications ...................................................................................... 15

II.
  Hypothesis .......................................................................................................... 17

III.
  Overall Aim ........................................................................................................ 17

IV.
  Specific Aims ..................................................................................................... 17

METHODS ........................................................................................................................ 19

I.
  Studies included ................................................................................................... 21

II.
  Overall inclusion and exclusion criteria ............................................................. 22

SURVIVAL STUDY ............................................................................................................ 23

EXERCISE TESTING STUDY .............................................................................................. 27

VENTRICULAR ARRHYTHMIAS STUDY ............................................................................. 31

I.
  Methodology ........................................................................................................ 33

II.
  Results ................................................................................................................. 38

1.-
  Characteristics of the Study Population ......................................................... 38

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CONTENTS
2.-
  Follow-up ....................................................................................................... 40

3.-
  ICD therapies .................................................................................................. 41

4.-
  Risk factors ..................................................................................................... 42

5.-
  Surgical cryoablation ...................................................................................... 45

6.-
  QRS duration and morphology ...................................................................... 47

7.-
  Events within one year of pulmonary valve replacement .............................. 47

8.-
  Survival .......................................................................................................... 48

9.-
  EP studies and ablation .................................................................................. 48

III.
  Discussion ........................................................................................................... 49

Surgical cryoablation .............................................................................................. 51

EP studies ............................................................................................................... 52

Limitations .............................................................................................................. 53

IV.
  Conclusion .......................................................................................................... 54

ECHOCARDIOGRAPHY STUDY .......................................................................................... 55

I.
  Methodology ........................................................................................................ 57

II.
  Results ................................................................................................................. 62

1.-
  Characteristics of the Study Population ......................................................... 62

2.-
  Echocardiography before and after pulmonary valve replacement ................ 63

3.-
  Ventricular-ventricular interactions before and after pulmonary valve
replacement ............................................................................................................. 67

4.-
  Outcomes ........................................................................................................ 68

5.-
  MRI analysis .................................................................................................. 70

III.
  Discussion ........................................................................................................... 72

Biventricular response after pulmonary valve replacement ................................... 72

Clinical implications ............................................................................................... 73

MRI 74

Limitations .............................................................................................................. 75

IV.
  Conclusion .......................................................................................................... 75

CONCLUSIONS ................................................................................................................. 77

I.
  Specific conclusions ............................................................................................. 79

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